Systemic Sclerosis (Scleroderma)

Systemic sclerosis (scleroderma) is characterized as a disorder that revolves around the
immune system (Badri & Hariz, 2020). The causes of the disease remain unknown, with
researchers still investing significantly to unearth the causes. However, genetic and environmental
factors play a role in the development of scleroderma (Badri & Hariz, 2020). Silica and other
organic solvents may increase one’s risk of getting the disease. The prevalence of the disease is
higher in individuals who have first-degree relatives with the disorder. In France, statistics
obtained in the year 2002 indicated that the disease had a prevalence rate of 158.3 per million
(Badri & Hariz, 2020).
Research indicates that the disorder is also associated with various abnormalities,
including fibrosis of the skin and internal organs (Rodríguez et al., 2018). Over the years,
researchers have attempted to study the resemblance between the disorder and other connective
tissue diseases. However, unlike other disorders, the clinical manifestation of the scleroderma
highly depends on the body parts involved (Rodríguez et al., 2018). The disorder has two distinct
categories, namely diffuse cutaneous and limited cutaneous. Research indicates that
approximately sixty percent of scleroderma patients suffer from limited cutaneous scleroderma.
The disorder mostly affects women compared to men. Diffuse cutaneous scleroderma is
characterized by thick or tight skin around the arms and above the elbow. The skin surrounding
the chest is also often tight (Rodríguez et al., 2018). Palpating the skin over several areas is the
main method that is used to detect abnormal skin thickening. On the other hand, patients with
limited cutaneous scleroderma have thick skin in areas below the elbows and the knees. The

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faces and areas above the elbow are not affected (Rodríguez et al., 2018). The thickening
develops gradually. Measures indicate that the skin continues to thicken over time.
The clinical manifestations vary from one patient to the next. Initial symptoms are related
to Raynaud’s phenomenon or chronic edema of hands and fingers. Initial symptoms are pain and
stiffness of fingers (Rodríguez et al., 2018). Some patients often report polyarthritis that is active
and migratory in nature. Almost all patients report Raynaud’s phenomenon. Raynaud’s
phenomenon alludes to vasoconstriction in areas such as fingers and the toes. The
vasoconstriction occurs in episodes. In some episodes, the vasoconstriction can extend to areas
of the nose and the ear lobes (Rodríguez et al., 2018). The attacks begin with pallor and are then
followed by cyanosis. Raynaud’s phenomenon is more frequent in women. As the disease
progresses, symptoms become worse. The edema that is often painless lasts several months
before the skin begins to tighten. The skin begins to feel indurated and stiff. As the disease
progresses, it becomes firmly bound to the various body structures. Painful induration of the
gums and the tongue are also common symptoms as the disease progresses.
Some patients experience tightening of the skin around the nose area, and this changes
the appearance of the nose. The skin that surrounds the neck also starts to tighten. In some
patients, hair loss is common (Paravina et al., 2015). Fingers become tapered, and recurrent
painful ulcers in the fingertips are also witnessed. The ulcers heal but develop from time to time
and are chronic. Cutaneous calcifications may form in some areas of the body, primarily around
the fingertips (Rodríguez et al., 2018). The calcifications may later develop ulcers. The disease
also affects the gastrointestinal system. Esophageal dysfunction is the most common
gastrointestinal disease. The disease is also associated with pulmonary impairment. About 13

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percent of scleroderma patients report pulmonary arterial hypertension. In addition, close to 90
percent of scleroderma report interstitial lung disease.

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References

Badri T., & Hariz J. (2020, January 28). Scleroderma (Systemic sclerosis) – StatPearls – NCBI
bookshelf. National Center for Biotechnology
Information. https://www.ncbi.nlm.nih.gov/books/NBK537335/
Paravina, M., Stanojević, M., Spalević, L., Ljubisavljević, D., Zlatanović, Z., & Popović, D.
(2015). CREST Syndrome-a limited form of systemic scleroderma: a case report and
literature review. Serbian Journal of Dermatology and Venereology, 7(3), 97-114.
Rodríguez-Franco, K., Miranda-Díaz, A. J., Hoyos-Restrepo, J. D., & Meléndez, G. L. (2018).
Systemic scleroderma: An approach from plastic surgery. Revista de la Facultad de
Medicina, 66(2), 237-245.